Safety and efficacy of capsular tension ring and capsular hook implantation for managing ectopia lentis in Marfan syndrome: real-world study.

PURPOSE: To evaluate the safety and efficacy of capsular tension ring and capsular hook (CTR-CH) implantation in Marfan syndrome (MFS) patients with ectopia lentis (EL). SETTING: Eye and ENT Hospital of Fudan University, Shanghai, China. DESIGN: Retrospective propensity score-matched cohort study. METHODS: This study included patients with MFS who had in-the-bag intraocular lens (IOL) implantation assisted by CTR-CH or modified CTR (MCTR). The safety analysis focused on the resurgery rate. The efficacy analysis compared the corrected distance visual acuity (CDVA) and the incidence of laser capsulotomy after propensity score matching (PSM). RESULTS: This study encompassed 148 eyes that had the CTR-CH procedure and 162 eyes that received MCTR implantation. In the CTR-CH group, the median age at the time of surgery was 5 years, with a mean follow-up duration of 1.81 ± 0.4 years. 5 eyes (3.38%) required a second surgery because of retinal detachment (2, 1.35%), IOL decentration (2, 1.35%), and CH dislocation (1, 0.68%). The resurgery rate was comparable with that of the MCTR group ( P = .486). After PSM, a total of 108 eyes were recruited in each group. Postoperative CDVA was significantly improved in both groups (both P < .001), but comparable between the groups ( P = .057). The posterior capsular opacification took place earlier ( P = .046) while the anterior capsular opacification required laser capsulotomy at a later stage ( P = .037) compared with the MCTR group. CONCLUSIONS: The CTR-CH procedure was a feasible, safe, and efficient approach for managing EL in patients with MFS.

Expanding the clinical spectrum and management of Traboulsi syndrome: report on two siblings homozygous for a novel pathogenic variant in ASPH.

BACKGROUND: Traboulsi syndrome is a very rare, syndromic form of ectopia lentis that is potentially sight-threatening at a young age. It is characterized by typical facial, skeletal and ocular signs. MATERIALS AND METHODS: Two siblings, born to consanguineous parents, with a clinical phenotype consistent with Traboulsi syndrome, underwent extensive ophthalmic imaging and exome-based genetic testing. Both were treated with unilateral clear lens extraction via a limbal approach. RESULTS: Two siblings, one male and one female, presented with systemic and ocular features consistent with Traboulsi syndrome. Lens subluxation was present in all 4fouraffected eyes, and spontaneous subconjunctival bleb formation was detected in one eye. This eye also showed evidence of keratoconus-related corneal thinning. The clinical diagnosis of Traboulsi syndrome was confirmed molecularly. A homozygous, novel, pathogenic nonsense variant was identified in exon 25 of the ASPH gene: c.2181_2183dup, p.(Val727_Trp728insTer). Excellent visual outcomes following clear lens extraction and postoperative rigid gas-permeable contact lens fitting were obtained. CONCLUSIONS: We expanded the genetic spectrum of Traboulsi syndrome with a novel frameshift variant in the ASPH gene. We showed that lensectomy followed by gas-permeable contact lenses is an efficient therapeutic approach to treat lens subluxation in Traboulsi syndrome. However, lifelong follow-up is crucial to avoid (late) postoperative complications.

Three-Year Outcomes of Cionni-Modified Capsular Tension Ring Implantation in Children Under 8 Years Old With Ectopia Lentis.

PURPOSE: This study evaluated visual outcomes and complications at 3 years post-implantation of a Cionni-modified capsular tension ring (MCTR) with an intraocular lens (IOL) in ectopia lentis patients ≤8 years old. DESIGN: Prospective clinical cohort study. METHODS: Included were 101 eyes from 57 patients <8 years of age, who underwent surgery for nontraumatic ectopia lentis between November 2015 and December 2016. Exclusion criteria were planned IOL fixation in the ciliary sulcus, severe intraoperative complications, and incomplete follow-up. All eyes received in-the-bag implantation of a posterior IOL and Cionni-MCTR. Posterior capsulectomy and anterior vitrectomy were performed through the pars plana in 23 eyes of children <5 years of age. Patients were examined at 1 day, and 1, 6, and 12 months, and at 2 and 3 years postoperatively. Outcome; measurements included best-corrected visual acuity (BCVA), intraocular pressure (IOP), IOL centration, and posterior capsule opacification (PCO). RESULTS: In all eyes, BCVA improved significantly after surgery, especially during the first 12 months (P < .05). Three years post-operatively, 44 eyes had BCVA 0.9 or better. Prophylactic Nd:YAG laser capsulotomy was performed 3 months post-surgery in 24 eyes; 34 eyes underwent this; procedure 6 months post-surgery because of PCO. A second surgery was; warranted in 4 eyes because of severe IOL decentration and combined anterior capsule contraction. No severe postoperative complications, such as retinal detachment or endophthalmitis, occurred. CONCLUSIONS: Implantation of in-the-bag IOL with Cionni MCTR is effective for visual rehabilitation in young children with ectopia lentis. A close follow-up of these patients is necessary to monitor IOL centration and stability.

Quantifying the Genetic Basis of Marfan Syndrome Clinical Variability.

Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder with considerable inter- and intra-familial clinical variability. The contribution of inherited modifiers to variability has not been quantified. We analyzed the distribution of 23 clinical features in 1306 well-phenotyped MFS patients carrying FBN1 mutations. We found strong correlations between features within the same system (i.e., ophthalmology vs. skeletal vs. cardiovascular) suggesting common underlying determinants, while features belonging to different systems were largely uncorrelated. We adapted a classical quantitative genetics model to estimate the heritability of each clinical feature from phenotypic correlations between relatives. Most clinical features showed strong familial aggregation and high heritability. We found a significant contribution by the major locus on the phenotypic variance only for ectopia lentis using a new strategy. Finally, we found evidence for the "Carter effect" in the MFS cardiovascular phenotype, which supports a polygenic model for MFS cardiovascular variability and indicates additional risk for children of MFS mothers with an aortic event. Our results demonstrate that an important part of the phenotypic variability in MFS is under the control of inherited modifiers, widely shared between features within the same system, but not among different systems. Further research must be performed to identify genetic modifiers of MFS severity.

Genetic mapping of autosomal recessive microspherophakia to chromosome 14q24.3 in a consanguineous Pakistani family and screening of exon 36 of LTBP2 gene.

Latent transforming growth factor beta binding protein 2 (LTBP2) plays a critical role in the development of connective tissue structure and function. Mutations in gene encoding LTBP2 are known to cause syndromic and a non-syndromic microspherophakia. Here, we present a 'first' report of genetic linkage of microspherophakia (MSP) to LTBP2 locus in a large consanguineous Pakistani family with four affected individuals in three loops. Using polymorphic microsatellite markers, haplotypes and linkage analysis, the diseased phenotype in MSP001 family was mapped to the LTBP2 gene. A maximum two point Logarithm of the odds (LOD) score of 4.16 was obtained with marker D14S284 at θ =0. Mutational analysis of exon 36 of LTBP2 using Sanger's sequencing did not reveal any previously reported mutations. Further analysis of the remaining exons are required to identify the causative variant.

Marfan syndrome: Evolving organ manifestations-A 10-year follow-up study.

The age-dependent penetrance of organ manifestations in Marfan syndrome (MFS) is not known. The aims of this follow-up study were to explore how clinical features change over a 10-year period in the same Norwegian MFS cohort. In 2003-2004, we investigated 105 adults for all manifestations in the 1996 Ghent nosology. Ten years later, we performed follow-up investigations of the survivors (n = 48) who consented. Forty-six fulfilled the revised Ghent criteria. Median age: females 51 years, range 32-80 years; males 45 years, range 30-67 years. New aortic root dilatation was detected in patients up to 70 years. Ascending aortic pathology was diagnosed in 93 versus 72% at baseline. Sixty-five percent had undergone aortic surgery compared to 39% at baseline. Pulmonary trunk mean diameter had increased significantly compared to baseline. From inclusion to follow-up, two patients (three eyes) developed ectopia lentis, four developed dural ectasia, four developed scoliosis, three developed incisional or recurrent herniae, and 14 developed hindfoot deformity. No changes were found regarding protrusio acetabuli, spontaneous pneumothorax, or striae atrophicae. The study confirms that knowledge of incidence and progression of organ manifestations throughout life is important for diagnosis, treatment, and follow-up of patients with verified or suspected MFS.

LTBP2-related "Marfan-like" phenotype in two Roma/Gypsy subjects with the LTBP2 homozygous p.R299X variant.

Recessive variants in LTBP2 are associated with eye-restricted phenotypes including (a) primary congenital glaucoma and (b) microspherophakia/megalocornea and ectopia lentis with/without secondary glaucoma. Nosology of LTBP2 pathology in humans is apparently in contrast with the consolidated evidence of a wide expression of this gene in the developing embryo. Accordingly, in previously published patients with LTBP2-related eye disease, additional extraocular findings have been occasionally reported and include, among others, high-arched palate, tall stature, and variable cardiac involvement. Anyway, no emphasis was put on such systemic manifestations. Here, we report two unrelated Roma/Gypsy patients first ascertained for a multisystem disorder mainly characterized by primary congenital glaucoma, complex congenital heart defect, tall stature, long fingers, skin striae and dystrophic scarring, and resembling Marfan syndrome. Heart involvement was severe with polyvalvular heart dysplasia in one, and transposition of great arteries, thoracic arterial tortuosity, polyvalvular heart dysplasia, and neo-aortic root dilatation in the other. Both patients were homozygous for the recurrent c.895C>T[p.(R299X)] variant, typically found in individuals of Roma/Gypsy descent with an eye-restricted phenotype. Our findings point out LTBP2 as responsible of a systemic phenotype coherent with the community of syndromes related to anomalies in genes involved in the TGFß-pathway. Among these disorders, LTBP2-related systemic disease emerges as a distinct condition with expanding prognostic implications and autosomal recessive inheritance.

PARS PLANA VITRECTOMY AND LENSECTOMY FOR ECTOPIA LENTIS WITH AND WITHOUT THE INDUCTION OF A POSTERIOR VITREOUS DETACHMENT.

PURPOSE: Posterior hyaloid removal during pars plana vitrectomy and lensectomy for ectopia lentis is commonly performed, but may increase the risk of intraoperative retinal breaks and postoperative retinal detachment. This study evaluated outcomes after pars plana vitrectomy and lensectomy with or without posterior hyaloid removal. METHODS: This retrospective observational cohort study included ectopia lentis cases that underwent pars plana vitrectomy and lensectomy (2005-2014), with or without intraoperative induction of a posterior vitreous detachment (PVD). The primary outcome was postoperative retinal detachment. The secondary outcomes were the incidence of iatrogenic retinal breaks, and change in visual acuity. RESULTS: Twenty-six cases were included. The posterior hyaloid was preserved intraoperatively in 11 cases (non-PVD group). In the remainder (15 cases), the vitreous was removed completely (PVD group). Postoperative retinal detachment occurred in 2 cases in each group (18.2% non-PVD vs.13.3% PVD, P = 0.7). Intraoperative breaks occurred more frequently in the PVD group (2 vs. 9 cases; P = 0.03). There was no difference in mean improvement in visual acuity (7 [PVD] vs. 3 [non-PVD] ETDRS lines; P = 0.2). CONCLUSION: The preservation of posterior hyaloid attachment during vitreolensectomy for ectopia lentis was associated with fewer iatrogenic retinal breaks. Postoperative retinal detachment did not seem to be influenced by the choice of surgical technique.

A cohort study of multiple families with FBN1 p.R650C variant, ectopia lentis, and low but not absent risk for aortopathy.

Marfan syndrome is a multisystem disease with cardiovascular, ophthalmologic, and skeletal features. Diagnosis is made clinically with emphasis on presence of aortic root dilation and ectopia lentis (EL). Most individuals meeting these criteria have a pathogenic variant in FBN1, usually unique or observed rarely. Individuals with EL alone may also have FBN1 pathogenic variants, and the risk for aortic disease is not well known. We identified a unique cohort of 31 individuals (mean age 29, range 2-78) from nine families ascertained by a proband with EL alone, who had the same FBN1 p.R650C variant. Comparison was made to individuals with Marfan syndrome (n = 103 from 97 families) at our institution. Those with the p.R650C variant had few skeletal features of Marfan syndrome. Age of onset of EL was later compared to others with cysteine variant changes. Aortic root dilation occurred in 4/16 (25%) of the p.R650C group versus 71/83 (86%) in the comparator group (p < 0.001) and dissection or replacement in 1/31 (3%) versus 20/103 (19%; p < 0.04). Aortic root Z scores were much lower in the p.R650C (0.34 ± 1.70) versus the comparator (2.99 ± 2.54; p < 0.0002). Kaplan-Meier failure curves for aortic root dilation demonstrated later age of onset and differed significantly for incidence rate ratio (comparator vs. p.R650C = 5.35, CI 1.84-21.17; p = 0.0001). Individuals with p.R650C predominantly have EL, but do have risk for aortic dilation at ages later than typical for Marfan syndrome in general and for cysteine changes specifically. Surveillance for aortic dilation is required but may occur less frequently.

Distribution of axial length in Chinese congenital ectopia lentis patients: a retrospective study.

BACKGROUND: Congenital ectopia lentis (CEL) usually leads to refractive error and may influence the axial length development. But few investigations have reported patient demographics and the distribution of axial length (AL) before surgery in Chinese pediatric patients with CEL. To describe the distribution of AL before surgery in CEL patients and its relationship with patients' demographics, such as age, Marfan syndrome, sex, and laterality. METHODS: This retrospective study reviewed 306 CEL patients from January 1, 2006 to December 31, 2015. One eye was randomly selected from each patient if both eyes were EL. The influences of Marfan syndrome, sex, and laterality to AL in different age subgroups were evaluated and compared. The differences of the AL between groups were assessed using the student t test or paired t-test. P-values less than 0.05 were considered statistically significant. RESULTS: Two hundred forty-seven eyes were enrolled. 58.3% of all the patients had binoculus EL, 70% of all the patients were male and 36% of all the patients were diagnosed with Marfan syndrome. The mean AL of EL patients was 25.1 ± 2.5 mm. There was no statistical difference in the AL between patients with and without Marfan syndrome, and in the AL between male and female patients. There was statistical difference in AL between the EL-affected eye and the unaffected eye in monocular EL patients younger than 12 years old. CONCLUSIONS: This study suggests that AL can be influenced by CEL, but the influence of CEL may be reduced after the age of 12 years old, which will likely provide a useful reference when considering the most appropriate time of surgery.

Anterior iris-claw intraocular lens implantation for the management of nontraumatic ectopia lentis: long-term outcomes in a paediatric cohort.

PURPOSE: To report the feasibility and long-term safety of lensectomy and iris-claw intraocular lens (IOL) implantation to treat children with severe ectopia lentis in a paediatric tertiary hospital. METHODS: Prospective cohort study of 21 eyes from 12 patients with severe ectopia lentis and visual acuity <20/63. All eyes underwent 23-gauge pars plana vitrectomy, lensectomy, iridectomy and Artisan IOL implantation in the anterior chamber with iris-claw enclavation via pars plana. Mean age at surgery was 8.0 ± 5.3 yo (range 3-17 years). A full ophthalmologic examination including best-corrected visual acuity (BCVA), biomicroscopy, intraocular pressure (IOP) measurement, fundus evaluation and central endothelial cell count (cECC) was performed pretreatment, at 3 months' postsurgery, and every 6 months thereafter. Ultrasound biomicroscopy (UBM) was performed 12 months after surgery. RESULTS: Mean follow-up was 39.3 ± 13.0 months. Best-corrected visual acuity (BCVA) (mean ± SD) improved from 0.91 ± 0.29 logMar preoperatively to 0.18 ± 0.23 logMar at final follow-up (p < 0.0001). Mean distance from the endothelium to the anterior IOL surface after surgery was 3.11 ± 0.61 mm. Postsurgically, cECC loss was 5.04% ± 9.58% with an annual cECC loss rate of 3.16% ± 4.46%. One patient developed IOL dislocation and retinal detachment after severe ocular contusion requiring vitrectomy, IOL refixation and gas tamponade. Another patient developed cystoid macular oedema, managed with intravitreal dexamethasone. CONCLUSION: This technique is both feasible and effective to manage severe ectopia lentis in children. Lifetime ophthalmic follow-up including cECC measurement, IOL position monitoring and fundus examination is mandatory in these patients.

Outcomes of Scleral-fixated Intraocular-lens in Children with Idiopathic Ectopia Lentis.

PURPOSE: The purpose of the study was to review the safety and stability of scleral-fixated intraocular lens (IOL) 2 years after implantation in children with idiopathic ectopia lentis (EL). METHODS: This retrospective case series enrolled children with EL managed between 2011 and 2015 at a tertiary eye hospital in the eastern of Saudi Arabia. Data were collected on age, gender, vision, isolated or syndromic pathology, intraoperative and postoperative complications, spherical equivalent refraction, and final best-corrected visual acuity (BCVA). RESULTS: The series included 18 eyes of 11 children with EL (6 males and 5 females). There were 7 bilateral and 4 unilateral cases. The median age was 3.5 years (25% quartile 1 year; range: 1-8 years). Preoperatively, fixation was absent in 3 eyes, 8 eyes were fixating but vision could not be recorded. In the remaining seven eyes, median distant visual acuity was 0.1 (25% quartile: 0.08). The median postoperative follow-up was 24 months (25% quartile, 7 months). Complications included two eyes with iris capture and one eye with lens subluxation requiring re-implantation. Postoperative BCVA was better than 20/60 in 15 (83%) eyes. At last follow-up, 6 eyes required myopic correction, 1 eye was emmetropic, and 11 eyes were hypermetropic. One eye with glaucoma was managed medically. CONCLUSION: Scleral fixated IOL in eyes of young children with EL had good visual outcomes and high stability. However, there is a high incidence of residual refractive error.

Outcomes after lensectomy for children with Marfan syndrome.

PURPOSE: To describe the long-term incidence of retinal detachment, intra- and postoperative complications, and visual and refractive outcomes of children with Marfan syndrome following lensectomy for ectopia lentis, with or without subsequent correction of aphakia using the Artisan aphakic iris-fixated anterior chamber intraocular lens. METHODS: The medical records of children treated at the Department of Ophthalmology, Children's University Hospital, Dublin, Ireland, from January 1, 1991, to December 31, 2011, were retrospectively reviewed. The primary outcome measure was rate of retinal detachment at final follow-up. Secondary outcomes included postoperative complications, endothelial cell count, visual acuity, refractive error, and postoperative refraction prediction error. RESULTS: A total of 30 eyes of 15 patients underwent lensectomy. Of these, 16 eyes of 8 patients subsequently underwent Artisan implantation. Mean follow-up was 13.8 ± 5.9 years after lensectomy and 4.1 ± 2.8 years after Artisan implantation. There were no cases of retinal detachment. Best-corrected visual acuity was 0.11 ± 0.14 (logMAR) post-lensectomy and 0.12 ± 0.19 post-Artisan implantation. Endothelial cell count of the pseudophakic group was 3109 ± 458 preoperatively and 2632 ± 592 postoperatively. Mean cell loss was 15.4%. One eye required repeat surgical peripheral iridectomy for pseudophakic pupillary block. One eye required re-enclavation of a dislocated Artisan IOL haptic; the same eye required anterior vitrectomy for removal of retained lens fragment from the original lensectomy. CONCLUSIONS: In this study cohort, there was no increased incidence of retinal detachment in children with Marfan syndrome and ectopia lentis in the setting of one or more prior intraocular procedures. A moderate rate of endothelial cell loss was observed.

Lensectomy, vitrectomy, and transvitreal ciliary body photocoagulation as primary treatment for glaucoma in microspherophakia.

Microspherophakia is a rare, bilateral developmental anomaly of the crystalline lens. It can occur in isolation or as a component of a familial disorder. It has been associated with the Weill-Marchesani syndrome and Marfan syndrome. Angle clousure glaucoma can occur in microspherophakia and is the primary cause of visual loss. We describe the management of 2 sisters with bilateral microspherophakia and advanced angle closure glaucoma.

Visual outcome and incidence of glaucoma in patients with microspherophakia.

INTRODUCTION: A number of ocular complications have been reported in microspherophakia. The literature however is limited to small case reports and the incidence of these complications is largely unknown. Our study describes a series of patients who presented to our hospital from 1998 to 2008. MATERIAL AND METHODS: Data on the clinical and surgical findings of patients presented to us from 1998 to 2008 with microspherophakia were retrieved from the medical records and the results analyzed. RESULTS: Thirty-six eyes of 18 patients were reviewed. The mean age at presentation was 16±10 years. All patients had varying degrees of lenticular myopia with a mean of -11.07±5.03 D. Glaucoma developed in 16 eyes (44.4%). Half of them had high IOP at presentation. Despite medical and surgical management IOP remained high in five eyes at the last follow-up. Sixteen eyes (44.4%) required lensectomy for dislocated crystalline lens. Lensectomy did not have any impact on the intraocular pressures. Homocysteinuria was the most common systemic association noted. CONCLUSION: Microspherophakia is associated with a high incidence of lenticular myopia, subluxation of the crystalline lens and glaucoma. Management of glaucoma is difficult with the IOP remaining high in spite of combined medical and surgical management.

Scleral-fixated capsular tension rings and segments for ectopia lentis in children.

PURPOSE: To report the short-term outcomes and complications of implantation of scleral-fixated capsular tension rings and/or capsular tension segments with intraocular lenses (IOL) in pediatric patients with ectopia lentis. DESIGN: Retrospective, observational case series. METHODS: Thirteen consecutive pediatric patients (19 eyes) underwent placement of in-the-bag IOL with either a Cionni modified capsular tension ring or a capsular tension segment in conjunction with a conventional capsular tension ring between January 1, 2009 and March 30, 2013 by 3 anterior segment surgeons at a single academic center. The scleral fixation suture was 9-0 polypropylene in 16 eyes and CV-8 Gore-Tex (expanded polytetrafluoroethylene) in 3 eyes. Outcome measures included change in corrected distance visual acuity (CDVA) and complications. RESULTS: The mean age was 10.2 years ± 4.8 (SD) and the median follow-up, 23.4 months. A Cionni modified capsular tension ring was implanted in 5 eyes and a capsular tension segment with an unsutured capsular tension ring was implanted in 12 eyes. In 2 eyes, capsular tension segment alone was placed. The mean CDVA at the final follow-up (0.10 ± 0.11 logMAR, 18 eyes) was significantly better than preoperatively (0.58 ± 0.26 logMAR, 15 eyes) (P < .001). The CDVA at the final follow-up was 20/40 or better in 18 eyes (94.7%). All IOLs were well centered. Posterior capsule opacification developed in 11 eyes (57.9%), 9 eyes (47.4%) required neodymium-yttrium-aluminum-garnet capsulotomy, and 3 eyes (15.8%) required pars plana vitrectomy and posterior capsulotomy. Other complications included broken suture (5.3%) (9-0 polypropylene at CTR eyelet, repaired with CV-8 Gore-Tex), conjunctival dehiscence (5.3%), suture exposure (5.3%) (trans-scleral 9-0 polypropylene), and vitreous strand at inferior paracentesis (5.3%). CONCLUSIONS: Implantation of in-the-bag IOL with either a Cionni modified capsular tension ring or a capsular tension segment in conjunction with a conventional capsular tension ring appears to be a safe and effective technique for visual rehabilitation in pediatric ectopia lentis.

Artisan iris-claw lenses for the correction of aphakia in children following lensectomy for ectopia lentis.

PURPOSE: To describe the results of Artisan iris-claw lens implants in children with aphakia following lensectomy for ectopia lentis. METHOD: We measured visual acuity, refractive error and endothelial cell counts and recorded complications in a group of children after insertion of Artisan iris-claw implants. RESULTS: Artisan implants were implanted in eight eyes of five children. Mean follow-up was 28 months (range: 4-58 months). The mean preoperative LogMAR best-corrected visual acuity was 0.21 ± 0.2, and postoperatively, mean LogMAR uncorrected visual acuity was 0.04 ± 0.09 (p=0.04). Mean endothelial cell count was 3312 ± 277 cells preoperatively and 2913 ± 268 cells postoperatively, representing a mean cell loss of 14.2% (p<0.001). Mean defocus equivalent was 11.38 ± 1.04 preoperatively and 2.34 ± 0.66 postoperatively. We encountered no postoperative complications in any of the eyes. CONCLUSION: Artisan iris-claw implants are safe and effective in the correction of aphakia in children following lensectomy for ectopia lentis, enabling good spectacle-free distance vision in 75% of eyes with no postoperative complications.

Corneal biomechanical properties and intraocular pressure measurement in Marfan patients.

PURPOSE: To compare the biomechanical properties of the cornea and intraocular pressure (IOP) between patients with Marfan syndrome and age-matched controls. SETTING: Departments of Ophthalmology and Genetics, Bakirkoy Maternity and Children Diseases Hospital, and Beyoglu Eye Education and Research Hospital, Istanbul, Turkey. DESIGN: Cross-sectional study. METHODS: This study comprised patients with Marfan syndrome (study group) and healthy individuals (control group). The study group was subdivided into patients with ectopia lentis and patients without ectopia lentis. In the right eye of each patient, the corneal hysteresis (CH), corneal resistance factor (CRF), Goldman-correlated IOP, and corneal-compensated IOP were recorded. RESULTS: Overall, the mean CH, CRF, Goldman-correlated IOP, and corneal-compensated IOP were not significantly different between the study group and the control group. The mean CH was 9.9 mm Hg ± 1.2 (SD) in study eyes with ectopia lentis and 11.2 ± 1.5 mm Hg in study eyes without ectopia lentis (P=.016); the mean CRF was 8.2 ± 1.8 mm Hg and 11.3 ± 1.9 mm Hg, respectively (P<.001). The mean Goldman-correlated IOP was 11.7 ± 2.7 mm Hg in study eyes with ectopia lentis and 16.2 ± 4.3 in study eyes without ectopia lentis (P=.003); the mean corneal-compensated IOP was 13.5 ± 4.1 mm Hg and 15.6 ± 3.8 mm Hg, respectively (P=.07). CONCLUSION: The CH, CRF, and Goldman-correlated IOP were significantly lower in the Marfan syndrome eyes with ectopia lentis than in the Marfan syndrome eyes without ectopia lentis.